U.S. Department of Health and Human Services

Clinical Research Section - Translational Hepatology Unit

Theo Heller, M.D., Chief


A selection of recent and significant publications can be viewed below.

Khangura SK, Kamal N, Ho N, Quezado M, Zhao X, Marciano B, Simpson J, Zerbe C, Uzel G, Yao MD, DeRavin SS, Hadigan C, Kuhns DB, Gallin JI, Malech HL, Holland SM, Heller T. Gastrointestinal Features of Chronic Granulomatous Disease Found During Endoscopy. Clin Gastroenterol Hepatol. 2015 Nov 3; 14 (2015 Nov 3; 14; 3):395-402.e5. [Full Text/Abstract]
Koh C, Canini L, Dahari H, Zhao X, Uprichard SL, Haynes-Williams V, Winters MA, Subramanya G, Cooper SL, Pinto P, Wolff EF, Bishop R, Ai Thanda Han M, Cotler SJ, Kleiner DE, Keskin O, Idilman R, Yurdaydin C, Glenn JS, Heller T. Oral prenylation inhibition with lonafarnib in chronic hepatitis D infection: a proof-of-concept randomised, double-blind, placebo-controlled phase 2A trial. Lancet Infect Dis. 2015 Jul 16; 15 (2015 Jul 16; 15; 10):1167-74. [Full Text/Abstract]
Hahn KJ, Ho N, Yockey L, Kreuzberg S, Daub J, Rump A, Marciano BE, Quezado M, Malech HL, Holland SM, Heller T, Zerbe CS. Treatment With Anakinra, a Recombinant IL-1 Receptor Antagonist, Unlikely to Induce Lasting Remission in Patients With CGD Colitis. Am J Gastroenterol. 2015 Jun; 110 (6; 110; 2015 Jun):938-9. [Full Text/Abstract]
Marciano BE, Spalding C, Fitzgerald A, Mann D, Brown T, Osgood S, Yockey L, Darnell DN, Barnhart L, Daub J, Boris L, Rump AP, Anderson VL, Haney C, Kuhns DB, Rosenzweig SD, Kelly C, Zelazny A, Mason T, DeRavin SS, Kang E, Gallin JI, Malech HL, Olivier KN, Uzel G, Freeman AF, Heller T, Zerbe CS, Holland SM. Common severe infections in chronic granulomatous disease. Clin Infect Dis. 2014 Dec 23; 60 (8; 60; 2014 Dec 23):1176-83. [Full Text/Abstract]
Jagasia MH, Greinix HT, Arora M, Williams KM, Wolff D, Cowen EW, Palmer J, Weisdorf D, Treister NS, Cheng GS, Kerr H, Stratton P, Duarte RF, McDonald GB, Inamoto Y, Vigorito A, Arai S, Datiles MB, Jacobsohn D, Heller T, Kitko CL, Mitchell SA, Martin PJ, Shulman H, Wu RS, Cutler CS, Vogelsang GB, Lee SJ, Pavletic SZ, Flowers ME. National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. The 2014 Diagnosis and Staging Working Group report. Biol Blood Marrow Transplant. 2014 Dec 18; 21 (2014 Dec 18; 21; 3):389-401.e1. [Full Text/Abstract]
Kuehn HS, Ouyang W, Lo B, Deenick EK, Niemela JE, Avery DT, Schickel JN, Tran DQ, Stoddard J, Zhang Y, Frucht DM, Dumitriu B, Scheinberg P, Folio LR, Frein CA, Price S, Koh C, Heller T, Seroogy CM, Huttenlocher A, Rao VK, Su HC, Kleiner D, Notarangelo LD, Rampertaap Y, Olivier KN, McElwee J, Hughes J, Pittaluga S, Oliveira JB, Meffre E, Fleisher TA, Holland SM, Lenardo MJ, Tangye SG, Uzel G. Immune dysregulation in human subjects with heterozygous germline mutations in CTLA4. Science. 2014 Sep 11; 345 (2014 Sep 11; 6204; 345):1623-7. [Full Text/Abstract]
Guay-Woodford LM, Bissler JJ, Braun MC, Bockenhauer D, Cadnapaphornchai MA, Dell KM, Kerecuk L, Liebau MC, Alonso-Peclet MH, Shneider B, Emre S, Heller T, Kamath BM, Murray KF, Moise K, Eichenwald EE, Evans J, Keller RL, Wilkins-Haug L, Bergmann C, Gunay-Aygun M, Hooper SR, Hardy KK, Hartung EA, Streisand R, Perrone R, Moxey-Mims M. Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr. 2014 Sep; 165 (165; 3; 2014 Sep):611-7. [Full Text/Abstract]
Guedj J, Rotman Y, Cotler SJ, Koh C, Schmid P, Albrecht J, Haynes-Williams V, Liang TJ, Hoofnagle JH, Heller T, Dahari H. Understanding early serum hepatitis D virus and hepatitis B surface antigen kinetics during pegylated interferon-alpha therapy via mathematical modeling. Hepatology. 2014 Aug 7; 60 (6; 2014 Aug 7; 60):1902-10. [Full Text/Abstract]
Harouni AA, Gharib AM, Osman NF, Morse C, Heller T, Abd-Elmoniem KZ. Assessment of liver fibrosis using fast strain-encoded MRI driven by inherent cardiac motion. Magn Reson Med. 2014 Jul 31; (2014 Jul 31). [Full Text/Abstract]
Heller T, Rotman Y, Koh C, Clark S, Haynes-Williams V, Chang R, McBurney R, Schmid P, Albrecht J, Kleiner DE, Ghany MG, Liang TJ, Hoofnagle JH. Long-term therapy of chronic delta hepatitis with peginterferon alfa. Aliment Pharmacol Ther. 2014 Jul; 40 (40; 2014 Jul; 1):93-104. [Full Text/Abstract]
Sakiani S, Koh C, Heller T. Understanding The Presence of False Positive Antibodies in Acute Hepatitis. J Infect Dis. 2014 Jun 18; 210 (2014 Jun 18; 210; 12):1886-9. [Full Text/Abstract]
Zhou Q, Yang D, Ombrello AK, Zavialov AV, Toro C, Zavialov AV, Stone DL, Chae JJ, Rosenzweig SD, Bishop K, Barron KS, Kuehn HS, Hoffmann P, Negro A, Tsai WL, Cowen EW, Pei W, Milner JD, Silvin C, Heller T, Chin DT, Patronas NJ, Barber JS, Lee CC, Wood GM, Ling A, Kelly SJ, Kleiner DE, Mullikin JC, Ganson NJ, Kong HH, Hambleton S, Candotti F, Quezado MM, Calvo KR, Alao H, Barham BK, Jones A, Meschia JF, Worrall BB, Kasner SE, Rich SS, Goldbach-Mansky R, Abinun M, Chalom E, Gotte AC, Punaro M, Pascual V, Verbsky JW, Torgerson TR, Singer NG, Gershon TR, Ozen S, Karadag O, Fleisher TA, Remmers EF, Burgess SM, Moir SL, Gadina M, Sood R, Hershfield MS, Boehm M, Kastner DL, Aksentijevich I. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med. 2014 Mar 6; 370 (2014 Mar 6; 10; 370):911-20. [Full Text/Abstract]
Hara K, Rivera MM, Koh C, Demino M, Page S, Nagabhyru PR, Rehermann B, Liang TJ, Hoofnagle JH, Heller T. Sequence analysis of hepatitis C virus from patients with relapse after a sustained virological response: relapse or reinfection?. J Infect Dis. 2014 Jan 1; 209 (209; 2014 Jan 1; 1):38-45. [Full Text/Abstract]
Rotman Y, Noureddin M, Feld JJ, Guedj J, Witthaus M, Han H, Park YJ, Park SH, Heller T, Ghany MG, Doo E, Koh C, Abdalla A, Gara N, Sarkar S, Thomas E, Ahlenstiel G, Edlich B, Titerence R, Hogdal L, Rehermann B, Dahari H, Perelson AS, Hoofnagle JH, Liang TJ. Effect of ribavirin on viral kinetics and liver gene expression in chronic hepatitis C. Gut. 2014 Jan; 63 (2014 Jan; 63; 1):161-9. [Full Text/Abstract]
Werner JM, Heller T, Gordon AM, Sheets A, Sherker AH, Kessler E, Bean KS, Stevens M, Schmitt J, Rehermann B. Innate immune responses in hepatitis C virus exposed healthcare workers who do not develop acute infection. Hepatology. 2013 Nov; 58 (58; 2013 Nov; 5):1621-31. [Full Text/Abstract]
Koh C, Turner T, Zhao X, Minniti CP, Feld JJ, Simpson J, Demino M, Conrey AK, Jackson MJ, Seamon C, Kleiner DE, Kato GJ, Heller T. Liver stiffness increases acutely during sickle cell vaso-occlusive crisis. Am J Hematol. 2013 Nov; 88 (2013 Nov; 11; 88):E250-4. [Full Text/Abstract]
Heller T, Werner JM, Rahman F, Mizukoshi E, Sobao Y, Gordon AM, Sheets A, Sherker AH, Kessler E, Bean KS, Herrine SK, Stevens M, Schmitt J, Rehermann B. Occupational Exposure to Hepatitis C Virus: Early T-Cell Responses in the Absence of Seroconversion in a Longitudinal Cohort Study. J Infect Dis. 2013 Sep; 208 (6; 2013 Sep; 208):1020-5. [Full Text/Abstract]
Alimchandani M, Lai JP, Aung PP, Khangura S, Kamal N, Gallin JI, Holland SM, Malech HL, Heller T, Miettinen M, Quezado MM. Gastrointestinal Histopathology in Chronic Granulomatous Disease: A Study of 87 Patients. Am J Surg Pathol. 2013 Sep; 37 (2013 Sep; 37; 9):1365-72. [Full Text/Abstract]
Safar Zadeh E, Lungu AO, Cochran EK, Brown RJ, Ghany MG, Heller T, Kleiner DE, Gorden P. The liver diseases of lipodystrophy: the long-term effect of leptin treatment. J Hepatol. 2013 Jul; 59 (59; 2013 Jul; 1):131-7. [Full Text/Abstract]
Hsu AP, Sowerwine KJ, Lawrence MG, Davis J, Henderson CJ, Zarember KA, Garofalo M, Gallin JI, Kuhns DB, Heller T, Milner JD, Puck JM, Freeman AF, Holland SM. Intermediate phenotypes in patients with autosomal dominant hyper-IgE syndrome caused by somatic mosaicism. J Allergy Clin Immunol. 2013 Jun; 131 (131; 6; 2013 Jun):1586-93. [Full Text/Abstract]
Alvarez-Downing MM, Kamal N, Inchauste SM, Khangura SK, Malech HL, Holland SM, Hughes MS, Heller T, Sherry RM. The role of surgery in the management of patients with refractory chronic granulomatous disease colitis. Dis Colon Rectum. 2013 May; 56 (56; 2013 May; 5):609-14. [Full Text/Abstract]
Hara K, Rivera MM, Koh C, Sakiani S, Hoofnagle JH, Heller T. Important factors in reliable determination of hepatitis C virus genotype by use of the 5' untranslated region. J Clin Microbiol. 2013 May; 51 (2013 May; 5; 51):1485-9. [Full Text/Abstract]
Fuss IJ, Friend J, Yang Z, He JP, Hooda L, Boyer J, Xi L, Raffeld M, Kleiner DE, Heller T, Strober W. Nodular regenerative hyperplasia in common variable immunodeficiency. J Clin Immunol. 2013 May; 33 (2013 May; 33; 4):748-58. [Full Text/Abstract]
Langan RC, Sherry RM, Avital I, Heller T, Henderson C, Holland SM, Freeman AF, Rudloff U. Safety of major abdominal surgical procedures in patients with hyperimmunoglobulinemia E (Job's syndrome): a changing paradigm?. J Gastrointest Surg. 2013 May; 17 (2013 May; 17; 5):1009-14. [Full Text/Abstract]
Kamal N, Chen C, Ling A, O'Donnell T, Heller T. Goodness gracious great balls of fire. BMJ Case Rep. 2013 Mar 15; 2013 (2013 Mar 15; 2013). [Full Text/Abstract]
Tana MM, Heller T. Autoantibodies in hepatitis C: red flag or bystander effect?. J Pediatr Gastroenterol Nutr. 2013 Mar; 56 (56; 2013 Mar; 3):243. [Full Text/Abstract]
Oppenheimer AP, Koh C, McLaughlin M, Williamson JC, Norton TD, Laudadio J, Heller T, Kleiner DE, High KP, Morse CG. Vanishing bile duct syndrome in human immunodeficiency virus infected adults: a report of two cases. World J Gastroenterol. 2013 Jan 7; 19 (19; 2013 Jan 7; 1):115-21. [Full Text/Abstract]
Fricker Z, Levy E, Kleiner D, Taylor JG 6th, Koh C, Holland SM, Heller T. Case series: biliary leak after transjugular liver biopsy. Am J Gastroenterol. 2013 Jan; 108 (108; 1; 2013 Jan):145-7. [Full Text/Abstract]
Gunay-Aygun M, Font-Montgomery E, Lukose L, Tuchman Gerstein M, Piwnica-Worms K, Choyke P, Daryanani KT, Turkbey B, Fischer R, Bernardini I, Sincan M, Zhao X, Sandler NG, Roque A, Douek DC, Graf J, Huizing M, Bryant JC, Mohan P, Gahl WA, Heller T. Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 2013 Jan; 144 (144; 1; 2013 Jan):112-121.e2. [Full Text/Abstract]
Eccleston JL, Koh C, Markello TC, Gahl WA, Heller T. An apparent homozygous deletion in maltase-glucoamylase, a lesson in the evolution of SNP arrays. Mol Genet Metab. 2012 Dec; 107 (2012 Dec; 107; 4):674-8. [Full Text/Abstract]
Fan X, Upadhyaya B, Wu L, Koh C, Santín-Durán M, Pittaluga S, Uzel G, Kleiner D, Williams E, Ma CA, Bodansky A, Oliveira JB, Edmonds P, Hornung R, Wong DW, Fayer R, Fleisher T, Heller T, Prussin C, Jain A. CD40 agonist antibody mediated improvement of chronic Cryptosporidium infection in patients with X-linked hyper IgM syndrome. Clin Immunol. 2012 May; 143 (2012 May; 2; 143):152-61. [Full Text/Abstract]
Leiding JW, Freeman AF, Marciano BE, Anderson VL, Uzel G, Malech HL, DeRavin S, Wilks D, Venkatesan AM, Zerbe CS, Heller T, Holland SM. Corticosteroid therapy for liver abscess in chronic granulomatous disease. Clin Infect Dis. 2012 Mar 1; 54 (2012 Mar 1; 5; 54):694-700. [Full Text/Abstract]
Kandiah E, Watts NR, Cheng N, Cardone G, Stahl SJ, Heller T, Liang TJ, Wingfield PT, Steven AC. Cryo-EM study of Hepatitis B virus core antigen capsids decorated with antibodies from a human patient. J Struct Biol. 2012 Jan; 177 (177; 1; 2012 Jan):145-51. [Full Text/Abstract]
O'Brien K, Font-Montgomery E, Lukose L, Bryant J, Piwnica-Worms K, Edwards H, Riney L, Garcia A, Daryanani K, Choyke P, Mohan P, Heller T, Gahl WA, Gunay-Aygun M. Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease. J Pediatr Gastroenterol Nutr. 2012 Jan; 54 (54; 1; 2012 Jan):83-9. [Full Text/Abstract]
Gunay-Aygun M, Turkbey BI, Bryant J, Daryanani KT, Gerstein MT, Piwnica-Worms K, Choyke P, Heller T, Gahl WA. Hepatorenal findings in obligate heterozygotes for autosomal recessive polycystic kidney disease. Mol Genet Metab. 2011 Dec; 104 (104; 4; 2011 Dec):677-81. [Full Text/Abstract]
Sandler NG, Koh C, Roque A, Eccleston JL, Siegel RB, Demino M, Kleiner DE, Deeks SG, Liang TJ, Heller T, Douek DC. Host response to translocated microbial products predicts outcomes of patients with HBV or HCV infection. Gastroenterology. 2011 Oct; 141 (4; 141; 2011 Oct):1220-30, 1230.e1-3. [Full Text/Abstract]
Arora M, Uzel G, Shea YR, Kleiner DE, Holland SM, Heller T. GI involvement in disseminated Mycobacterium genavense: endoscopy and histology. Gastrointest Endosc. 2011 Sep; 74 (74; 2011 Sep; 3):688-90. [Full Text/Abstract]
Noureddin M, Kleiner DE, Gharib AM, Bratslavsky G, Heller T. To resect or not to resect? That is the question. Telangiectatic adenoma. Gut. 2011 Sep; 60 (2011 Sep; 60; 9):1177, 1235. [Full Text/Abstract]
Shin EC, Park SH, Demino M, Nascimbeni M, Mihalik K, Major M, Veerapu NS, Heller T, Feinstone SM, Rice CM, Rehermann B. Delayed induction, not impaired recruitment, of specific CD8⁺ T cells causes the late onset of acute hepatitis C. Gastroenterology. 2011 Aug; 141 (2; 141; 2011 Aug):686-95, 695.e1. [Full Text/Abstract]
Weinstein DH, Childs R, Heller T. Giant duodenal ectasia. Clin Gastroenterol Hepatol. 2011 Jun; 9 (6; 2011 Jun; 9):A24. [Full Text/Abstract]
Feld JJ, Modi AA, El-Diwany R, Rotman Y, Thomas E, Ahlenstiel G, Titerence R, Koh C, Cherepanov V, Heller T, Ghany MG, Park Y, Hoofnagle JH, Liang TJ. S-adenosyl methionine improves early viral responses and interferon-stimulated gene induction in hepatitis C nonresponders. Gastroenterology. 2011 Mar; 140 (2011 Mar; 140; 3):830-9. [Full Text/Abstract]
Veerapu NS, Raghuraman S, Liang TJ, Heller T, Rehermann B. Sporadic reappearance of minute amounts of hepatitis C virus RNA after successful therapy stimulates cellular immune responses. Gastroenterology. 2011 Feb; 140 (2; 2011 Feb; 140):676-685.e1. [Full Text/Abstract]
Kuhns DB, Alvord WG, Heller T, Feld JJ, Pike KM, Marciano BE, Uzel G, DeRavin SS, Priel DA, Soule BP, Zarember KA, Malech HL, Holland SM, Gallin JI. Residual NADPH oxidase and survival in chronic granulomatous disease. N Engl J Med. 2010 Dec 30; 363 (363; 27; 2010 Dec 30):2600-10. [Full Text/Abstract]
Uzel G, Orange JS, Poliak N, Marciano BE, Heller T, Holland SM. Complications of tumor necrosis factor-α blockade in chronic granulomatous disease-related colitis. Clin Infect Dis. 2010 Dec 15; 51 (2010 Dec 15; 12; 51):1429-34. [Full Text/Abstract]
Chetty-John S, Piwnica-Worms K, Bryant J, Bernardini I, Fischer RE, Heller T, Gahl WA, Gunay-Aygun M. Fibrocystic disease of liver and pancreas; under-recognized features of the X-linked ciliopathy oral-facial-digital syndrome type 1 (OFD I). Am J Med Genet A. 2010 Oct; 152A (152A; 2010 Oct; 10):2640-5. [Full Text/Abstract]
Kucirka LM, Farzadegan H, Feld JJ, Mehta SH, Winters M, Glenn JS, Kirk GD, Segev DL, Nelson KE, Marks M, Heller T, Golub ET. Prevalence, correlates, and viral dynamics of hepatitis delta among injection drug users. J Infect Dis. 2010 Sep 15; 202 (6; 202; 2010 Sep 15):845-52. [Full Text/Abstract]
von Rosenvinge EC, Koh C, Holland SM, Heller T. When to leave a stone unturned ... Gut. 2010 Aug; 59 (8; 59; 2010 Aug):1036, 1100. [Full Text/Abstract]
Feld JJ, Lutchman GA, Heller T, Hara K, Pfeiffer JK, Leff RD, Meek C, Rivera M, Ko M, Koh C, Rotman Y, Ghany MG, Haynes-Williams V, Neumann AU, Liang TJ, Hoofnagle JH. Ribavirin improves early responses to peginterferon through improved interferon signaling. Gastroenterology. 2010 Jul; 139 (2010 Jul; 139; 1):154-62.e4. [Full Text/Abstract]
Sakiani S, Koh C, Heller T. Glowing in the dark: not always a bad thing. Gastroenterology. 2010 Mar; 138 (138; 3; 2010 Mar):e7-8. [Full Text/Abstract]
Ahlenstiel G, Titerence RH, Koh C, Edlich B, Feld JJ, Rotman Y, Ghany MG, Hoofnagle JH, Liang TJ, Heller T, Rehermann B. Natural killer cells are polarized toward cytotoxicity in chronic hepatitis C in an interferon-alfa-dependent manner. Gastroenterology. 2010 Jan; 138 (138; 2010 Jan; 1):325-35.e1-2. [Full Text/Abstract]